Pigmented Lesions.

Oral pigmented lesions have a wide range of clinical presentations, some of which correlate with cutaneous pigmented lesions. This article highlights these correlates and underscores important differences that can potentially have clinical impact. Moreover, given a nonspecific presentation of an oral pigmented lesion, the article provides a reference to aid clinicians with differential diagnoses based on clinical features. This article is an overview of pigmented lesions of the oral cavity, including localized reactive pigmented lesions, neoplastic pigmented lesions, and pigmented lesions as sequelae of a systemic disease.

An unusual tumour causing neonatal respiratory distress.

PROBLEM: We present the case of a term neonate referred shortly after birth because of breathing and feeding difficulties. METHODOLOGY: Fiber-endoscopic examination of the nasal cavity showed a pendulating mass in the nasopharynx. RESULTS: A complete surgical resection was performed and the baby recovered completely. Microscopic examination of the mass showed an overlying non-keratinized squamous cell lining with an atypical cell population in some fragments. Histological features were compatible with a high-grade epithelial tumour like a midline carcinoma, but a final diagnosis of a salivary gland anlage tumour was established. CONCLUSION: Flexible fiber endoscopy is the method of choice for examining the nasal passages and oropharynx in neonates with respiratory distress. Congenital salivary gland anlage tumour is a rare cause of neonatal nasal obstruction; it is benign and complete excision results in a cure. Histologically, it may mimic a malignant tumour owing to the high mitotic index.

Melanotic neuroectodermal tumor of infancy: report of one calvarial lesion with T1 shortening and one maxillary lesion.

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor which commonly involves the mandible, maxilla and calvarium. We report two infants with this rare tumor. T1 shortening due to melanin content has been rarely described in MNTI. The calvarial MNTI showed T1 shortening which was helpful in diagnosis.

Salivary gland anlage tumor in a neonate presenting with respiratory distress: radiographic and pathologic correlation.

We present a case of congenital salivary gland anlage tumor (SGAT) of the nasal septum in a 2-week-old infant who had difficulty breathing through her nose since birth. CT and MR imaging demonstrated a circumscribed mass within the nasal cavity that did not communicate with the intracranial compartment. Differential diagnosis and clinical significance of recognizing this rare lesion are reviewed.

Síndrome de Gorlin (Síndrome neovoide basocelular) / Gorlin syndrome. (Nevoid basal cell carcinoma syndrome)

Caso clínico: Varón de 77 años con enfermedad de Parkinson y demencia senil. Presentaba múltiples carcinomas basocelulares faciales y ectropión en ojo izquierdo. Comenzó con insuficiencia respiratoria y fue diagnosticado de ameloblastoma en fosa nasal izquierda e intervenido quirúrgicamente. Discusión: El síndrome de Gorlin es una enfermedad autosómica dominante caracterizada por carcinomas basocelulares, anomalías esqueléticas y del sistema nervioso. Su pronóstico depende de la evolución de las lesiones malignas. Es importante sospechar un síndrome de Gorlin en pacientes jóvenes con múltiples carcinomas basocelulares o en pacientes que acuden al oftalmólogo con estas lesiones a nivel palpebral, ya que su seguimiento es fundamental

Clinical case: A 77 year-old male patient with Parkinson’s disease and senile dementia had many facial basal cell carcinomas and an ectropion of the left eye. When he experienced respiratory difficulty he was diagnosed to have an ameloblastoma in left nostril requiring surgery. Discussion: Gorlin syndrome is an autosomal dominant condition characterized by basal cell carcinomas, and skeletal and neurological anomalies. The presence of multiple basal cell carcinomas on the eyelids in a child or in a young patient should alert ophthalmologists to the possibility of this syndrome (Arch Soc Esp Oftalmol 2008; 83: 321-324)

Benign melanocytic tumor in infancy: discussion on a rare case and review of the literature.

Meningeal melanocytoma is an infrequent neoplasm of the central nervous system (CNS), especially in childhood and infancy. It was first described as an entity different from pigmented meningiomas and schwannomas in 1972, and few cases have been published so far. In this article, a 5-month-old male patient with meningeal melanocytoma is presented. This midline lesion was localized in the posterior fossa and manifested by hydrocephalus. The entire dural origin and extradural growing pattern in addition to the destruction of the adjacent occipital bone were the unexpected presentations since these tumors usually tend to locate on leptomeninges and to extend into the adjacent neural compartment rather than the outside. On the other hand, this case is the only one which had identical lesions in both surrenal glands and the left renal capsule, the structures containing neural-crest-derived cells outside the CNS. The prognostic criteria, differential diagnosis and its embryological aspects are discussed with an extensive review of the related existing literature.